AAC Treatment for Persons with Primary Progressive Aphasia (PPA)

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AAC Treatment for Persons with Primary
Progressive Aphasia (PPA)

• Melanie Fried-Oken
• Professor, Neurology, BME, ENT Pediatrics
• Oregon Health Science University
• Portland, Oregon

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Goals for afternoon

• Participants will
• Become familiar with language symptoms of PPA
• Formulate AAC goals for language intervention in
  adults with PPA
• Understand clinical research implications for
  intervention decisions related to adults with PPA.

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• We are the raft after the wreck. (LaPointe)
• AAC are the paddles for the raft. (MFO)

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What is PPA?

• A degenerative language disorder.
• A language disorder that does not easily fit
  into the classical aphasia typology.
• A syndrome, often followed by cognitive decline,
  that has been described with 3 variants.

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Diagnostic Criteria for PPAMesulam, M. Annals of
Neurology, 49 (4), April, 2001

• Insidious onset and gradual loss of word finding,
  object-naming or word-comprehension skills in
  spontaneous conversation

• ADL limitations attributable to language
  impairment, for at least 2 yrs after onset

• Absense of significant apathy, disinhibition,
  forgetfulness for recent events, visuospatial
  impairment, visual recognition deficits or
  sensory-motor dysfunction within initial 2 yrs of
  L impairment

• Intact premorbid language skills

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• Acalculia ideomotor apraxia may be present in
  first 2 yrs.

• Other domains possibly affected during 2 yrs, but
  language most impaired fn.

• Absence of specific causes (i.e., stroke, tumor,
  infection, metabolic disorder) on neuroimaging.

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Dx Characteristics

• Age of onset 40-75 years old, mean onset age of
  60 years.
• Preponderance of male patients

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PPA is a clinical syndrome which may overlap with

• Alzheimers disease
• Frontotemporal dementia
• Corticobasal degeneration
• Dementia-lacking-distinctive-histology (DLDH)
• CJD
• ALS
• ACD (Asymmetric cortical degeneration Caselli,
  1995)
• Picks disease
• Kertesz Munoz, Amer. J. of Alzheimers Disease.
  (2002), 17(1).

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3 variants of PPA

• Nonfluent progressive aphasia (NFPA)
• PPA with agrammatism
• Semantic dementia (SD)
• Fluent progressive aphasia
• Logopenic progressive aphasia (LPA)
• PPA with comprehension deficits

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NFPA nonfluent progressive aphasia (most common
type of PPA in an AAC clinic)

• Anomia or trouble thinking of or remembering
  specific words when talking or writing.
• Initial empty speech with preserved melody and
  fluency but little information.
• Slow, hesitant and labored speech frequently
  punctuated by long pauses and filler words, early
  symptoms of agrammatism.
• Simplification (generic words for specific
  concept)
• Circumlocutions
• Substitution by fillers (thing,
  Whachamacallit)
• Phonemic paraphasias
• Marked increase in speech errors, early symptoms
  of a progressive apraxia of speech.
• Relatively preserved single-word comprehension
  with later difficulty comprehending complex
  syntactic structures.
• Stronger oral reading that generative language
  skills.

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Progression of disease varies

• Yes/No confusion for responses
• Apraxia of Speech
• Agrammatism -gt Mutism
• Written language generation often mimics spoken
  language generation.

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SD (semantic dementia)

• Fluent, grammatical speech
• Confrontation naming deficits (often word
  knowledge can be accessed through
  visuo-perceptual route)
• Surface dyslexia
• Deficits in word comprehension (2-way naming
  problems) In time, even the most common words
  fail to be decoded and the comprehension of
  conversation becomes impossible, although visual
  recognition of objects and faces remains
  relatively preserved (Mesulum, 2001).
• Later connected speech includes neologisms and
  semantic paraphasias.

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LPA Logopenic progressive aphasia

• Word finding difficulties within fluent speech
• Decreased output but relatively preserved syntax
  and phonology
• Combination of the dysfluencies of NFPA with the
  semantic comprehension deficits of SD.

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• The principal function of language is to label,
  categorize, and communicate thoughts through the
  mediation of arbitrary symbols (words).
• Damage to any part of the language network can
  interfere with word usage and word finding. Such
  anomic deficits provide sensitive markers for
  dysfunction within the language network.
  Consequently, anomia emerges as a nearly
  universal finding in the early stages of PPA.
• Mesulam, M. (2001). Primary progressive aphasia.
  Annals of Neurology 49 425-432.

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Some dx drama..

• Within neurologic syndrome identification
• PPA falls under the Neary Criteria for FTLD
  Fronto-temporal lobar dementia (Neary, D, et al.
  Neurology, 1998).
• Kertesz et al consider it a variant of Picks
  disease (Kertesz, A, et al. Neurology, 2000).
• Confusion on term semantic dementia. 3 distinct
  variants (Gorno-Tempini, ML, et al. Annals of
  Neurology, 2004).

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Concomitant symptoms reported in association with
PPA Speech and LanguageRogers Alarcon (2000).
AAC for Adults with Acquired Neurological
Disorders.

• Agrammatism
• Alexia
• Anomia
• Apraxia of speech
• Dysarthria
• Dysgraphia
• Dysprosodia
• Echolalia palilalia
• Hoarse voice quality
• Impaired aud. Comp.
• Impaired repetition
• Impaired spelling
• Impaired syntax

• Mutism
• Neologisms
• Phonemic paraphasias
• Pure word deafness
• Reduced sentence length
• Semantic paraphasias
• Slow speaking rate
• Staccato speech
• Stuttering
• Telegraphic Speech
• Verbal hesitancy long phrases

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Concomitant symptoms reported in association with
PPA Cognitive SensoryRogers Alarcon (2000).
AAC for Adults with Acquired Neurological
Disorders.

• Acalculia
• Amusia
• Dressing apraxia
• Hemianopia
• Hypethesia
• Impaired executive function
• Impaired nonverbal functions

• Impaired verbal memory
• Perseverative behavior
• Poor recall
• Prosopagnosia
• Short-term memory deficits
• Visual agnosia

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Proposed Stages of Intervention during the
Neurodegenerative Language ProcessNFPA

• No noticeable interference with generative
  language but some word finding problems
• Detectable language lapses with hesitations and
  dysfluencies
• Reduction in language use leading to behavioral
  strategies and introduction of low tech AAC
  (circumlocutions paraphasias simplification
  agrammatism)
• Use of AAC tools and other techniques
• No functional language.

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PPA presents a clinical conundrum

• Language behavior is truly variable, and we dont
  know if we are looking at impairment of
  linguistic competence or performance.

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Communication Treatment Goals

• 1 To compensate for progression of language
  loss (NOT stimulate the language system to regain
  skills).
• 2 To start early. Begin compensatory treatment
  as soon as possible. Be proactive so patient can
  learn to use communication strategies and tools.
• 3 To include primary communication partners in
  all aspects of training, with outreach to
  multiple partners.

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The Treatment Challenges

• To put the patients residual lexicon visually in
  front of him so that the patient can participate
  in daily activities as language skills decline.
• To engineer the environment to support successful
  communication.

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This is where AAC comes in.You should be
asking yourself
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• Where, on the natural communication continuum,
  can we intervene first with AAC treatment for the
  person with PPA and their partners?
• How do AAC strategies and devices change with
  progression of the syndrome?

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Enter REKNEW-AD

• REKNEW-AD
• Reclaiming
• Expressive
• Knowledge
• in Elders
• With
• Alzheimers disease

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Premise for REKNEW-AD research

• Pairing external aids with familiar and spared
  skills (such as page turning, reading aloud,
  personal information) maximizes a persons
  opportunity for success.
• These skills are based on intact procedural and
  autobiographical memory.
• Symbolic representations may serve as semantic
  primes to stimulate lexical retrieval during
  conversation in moderate Alzheimers disease
  (modAD).

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• 3-year research project funded by NIH and NIDRR,
  with pilot funds from the Oregon Tax Check-off
  Funds.
• PIs Melanie Fried-Oken and Charity Rowland
• Study 1 Question Do AAC supports improve
  conversation by individuals with moderate
  Alzheimers disease?

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Method

• Determine subjects preferred topic and
  vocabulary
• Develop communication board
• Conduct 9 10-minute videotaped conversations in
  homes with and without the AAC device
  (conversational condition randomly
  assigned/visit).

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Study 1 subjects with moderate Alzheimers
disease (N30)Diagnosis of probable or possible
AD by a board certified neurologistVision and
hearing within functional limits English as
primary languageExclude those with prior
neurological diagnoses or communication disorders.
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Clinical messageAAC WITHOUT TRAINING IS NO AAC
AT ALL!
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Study 2 Question Do AAC supports combined with
spaced retrieval priming exercises improve
conversation by individuals with moderate
Alzheimers disease?
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• Added PPA as more patients showed up in the OHSU
  AAC clinic.

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Wayne

• 62 year old man
• Retired HVAC technician
• Completed high school
• In-line flying airplane hobbyist (owned a hobby
  store for a while with his wife)
• Lives in urban Portland, Oregon with his wife.

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Language screening

• BDAE Complex ideational material (85, errors on
  last 2 paragraphs) offered yes/no cards for
  response verification
• BDAE Writing to dictation 5/10
• RCBA Functional Reading subtest 8/10.

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FLCI 73/82(between mild moderate)

• Receptive score 39. Expressive score 34.
• Could not name half of pictures shown (hanger,
  harmonica, stethoscope, compass).
• Could not answer 2/3 open-ended questions (where
  would you like to go on a trip what's your
  favorite holiday).
• Could not successfully write sentence about self
  (wrote nothing).
• Could not write all words to dictation
  (harmonica, compass, knocker, stethoscope)
• Could not pantomime 2 of 9 pictures shown
  (harmonica hanger)

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MMSE- 13/30

• Could not name year, season, date, day, month, or
  county of residence.
• Could not spell WORLD backwards.
• Could not recall 3 words from earlier in exam.
• Could not successfully repeat, "No ifs ands or
  buts."
• Could not write a sentence (free-writing about
  anything).

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CDR (wife report)- 1 (mild)

• Memory only highly learned material retained
  new material rapidly lost
• Orientation Severe difficulty with time
  relationships Usually disoriented to time
• Judgment problem solving Severely impaired in
  handling problems, similarities, and differences
• Community affairs unable to function
  independently at these activities although may
  still be engaged appears normal to casual
  inspection
• Home hobbies mild but definite impairment of
  function at home more difficult chores
  abandoned more complicated hobbies and interests
  abandoned
• Personal care needs prompting

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SIB 3 (out of 4)

• He could not answer "what is this" when shown the
  picture of the spoon (although he could with the
  cup). He could successfully pantomime how to use
  both the spoon and the cup.

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SALT variables to considerN12 control and 6 AAC
conversations

• Total utterances
• non-productive utterances
• explanatory utterances
• MLU
• abandoned utterances
• interrupted utterances
• Mean turn length in utterances
• Mean turn length in words
• Total words
• Type/token ratio
• on-target words
• words first mentioned

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Control vs. experimental 10-minute
conversationsUtterance variables
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Lexical variables
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What do the numbers mean?

• With AAC support, we see more words and
  utterances.
• With AAC support, we see longer turns (in words
  and utterances).
• With AAC support, we see more non-productive,
  abandoned and interrupted utterances, but these
  may be permitted because of joint references.
• The AAC support provides a non-verbal symbolic
  joint reference that reduces TTR and increases 1
  word responses.

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Tool use will vary according to the demands of
the situation (performance??) and the users
abilities (L competence??)

• Consider language use variables
• Communication needs
• Purpose of communication
• Activity restrictions or opportunities
• Level of communicator (independent, transitional,
  partner-dependent)

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Visual representation for language

• Personal dictionary
• Calendars Clocks
• Maps
• PDAs
• Family trees
• Pictures or photographs
• Drawings
• Remnant bags
• Augmented Input
• Continuum lines for conversation
• Hate __________________________ Adore

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Low tech tools

• Customized communication boards
• Customized brag books
• Remnant bags/boxes
• Single message devices
• Talking photo albums

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Personalized goals and tx

• What would you like to be doing today that you
  are not doing? (Lasker)
• What goals would you like to pursue?
• How do you feel about your day? (Murphy,2000)
• How do you feel about your living situation
  (QofL) (Murphy and Gray, 2006)

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Talking Mats www.talkingmats.com
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What is Talking Mats?

• A visual framework using picture symbols to help
  people with communication impairments to interact
  more effectively.
• A supported means to help individuals with choice
  making, goal setting, sharing opinions, and
  directing individual options.
• An interactive resource that uses 3 sets of
  picture symbols (topic, options, visual scale)
  with a textured mat.
• Presents topics in a structured, consistent and
  visual means for both comprehension and
  expression.

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High tech tools

• Dynamic display devices with customized messages
• Dynavox V or M3
• Words Say It Sam Tablet SM1

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• Digitized devices with hard copy pages (Saltillo
  Bluebird II or VocaFlex)
• Talking Photo Album (Augmentative Communication,
  Inc.)

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Messaging

• What messages to include in tools?
• Svoboda 100 autobiographical memories for elders
• Story telling 87 of adult conversation is
  reminiscence and chatting

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Partner training is an essential component of AAC
for persons with PPA.

• To identify vocabulary for external lexicon.
• To support use of tools in familiar communication
  settings.
• To identify new opportunities for communication
  with tools.
• To offer or confirm choices.
• To initiate conversation during late stages of
  PPA.

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Rogers, MA Alarcon, NB. (1998). Dissolution of
spoken language in primary progressive aphasia.
Aphasiology.
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www.brain.northwestern.edu/PPA

• PPA Newsletters from 1996 (on line)
• Join mailing lists
• Connect to PPA databases
• Clinician search and database
• PPA literature database
• Question and answer archive
• PPA Family Support Group
• maintained by The Cognitive Neurology
  Alzheimers Disease Center at Northwestern
  University, Dr. M. Mesulam

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AAC treatment in PPA Workgroup discussion
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Tx dependent on level of communication ability

• Independent communicator
• Transitional communicator
• Partner-dependent communicator

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Communication considerations

• Behavioral strategies
• Low tech techniques
• High tech techniques
• Partner-based strategies

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Jack

• 70 years old
• Retired aeronautics engineer
• Built minutemen missiles and was consultant to
  U.S. military and private industry
• Moved to Portland, Oregon 1 year earlier, to be
  near daughters family

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Test scores

• BDAE Complex ideational material-100 with
  yes/no cards
• BDAE Writing to dictation 8/10 with
  recognition of errors
• RCBA Functional reading subtest 10/10
• MMSE- 29 (out of 30) Only recited 2/3 words
  remembered from a few questions earlier in the
  exam.
• CDR- 0 (no impairment)
• FLCI-- 80/82 Expressive score 39. Receptive
  score 41. (Couldn't name stethoscope when shown
  a picture, did not correctly spell a few words to
  dictation (harmonica, knocker, stethoscope), and
  when asked to write a sentence about himself, he
  wrote, "I a minuteman millse guidance expert.)
• SIB- 4 (out of 4)

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Recent references

• King, J. Alarcon, N., Rogers, MA (2007).
  Primary progressive aphasia. In DR Beukelman, K
  Garrett, and K Yorkston (Ed.). Augmentative
  communication strategies for adults with acute or
  chronic medical conditions. Baltimore Paul H
  Brookes Publishing.
• Murphy, J. (2000). Enabling people with aphasia
  to discuss quality of life. British Journal of
  Therapy and Rehabilitation. 7(11) 454-458.
• Rogers, MA Alarcon, N. (2000). Proactive
  management of primary progressive aphasia. In DR
  Beukelman, KM Yorkston, J. Reichle (Ed.). AAC
  for Adults with Acquired Neurological Disorders.
  Baltimore Paul H Brookes Publishing.

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ACKNOWLEDGEMENTS

• Layton Center for Aging and Alzheimers Disease
  Research, Portland, Oregon, USA
• NIH/NICHD/NCMRR award 1 R21 HD47754-01A1
• DOE/NIDRR award H133G040176

Well, I could use this board to talk from
breakfast to hell and back!